Current and Upcoming Treatment Modalities in Myasthenia Gravis

Purpose: This case report describes myasthenia gravis-like symptoms after treatment with a programmed cell death 1 inhibitor, pembrolizumab, the treatment modalities utilized, and associated patient outcomes. Summary: A 76-year old male treated with pembrolizumab for palliative therapy for metastatic melanoma presented with increasing weakness, neck pain, diplopia in the left eye, abducens palsy, periorbital edema, and decreased appetite. The patient was diagnosed with acetylcholine receptor antibody (AChR) negative myasthenia gravis. The patient was started on prednisone 1 mg/kg/day, followed by pyridostigmine 60 mg by mouth 3 times a day, and IVIg for 5 days. Due to minor improvements in myasthenia gravis symptoms, 5 cycles of plasmapheresis were ordered. The patient was successfully treated for aspiration pneumonia after cardiopulmonary arrest. On day 28, the patient was diagnosed with ventilator associated pneumonia and received appropriate therapy. Due to ICU agitation and delirium, VAP, and long duration of treatment, the patient requested withdrawal of care and passed. Conclusion: Programmed cell death inhibitors, such as pembrolizumab, can provide great benefit to patients but can also be associated with rare but serious adverse events. With new reports of MG after use, providers should continually weigh the benefits versus harm in using these products and monitor patients closely for such adverse events.

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An 80-Year-Old Myasthenia Gravis Patient with Worsening Weakness

10.1093/med/9780190491901.003.0031 ◽

2016 ◽

Author(s):

Jeffrey A. Cohen ◽

Justin J. Mowchun ◽

Victoria H. Lawson ◽

Nathaniel M. Robbins

Keyword(s):

Myasthenia Gravis ◽

Respiratory Insufficiency ◽

Signs And Symptoms ◽

Treatment Modalities ◽

Positive Pressure ◽

Myasthenic Crisis ◽

Effective Management ◽

Extreme Caution ◽

Single Breath ◽

Steroid Sparing

In myasthenia gravis, weakness and respiratory insufficiency can occur quickly. It is important for the treating physician to recognize this and institute treatment rapidly. Increasing weakness of the neck may herald impending respiratory insufficiency. The single breath count is an easy way ti assess ventilatory function. Because of bulbar weakness and increasing secretions usually bi-level positive pressure airway pressure is used with extreme caution. Intubation with effective management if the airway is preferred. Differentiation of myasthenic crisis from cholinergic crisis is explained; although cholinergic crisis is relatively uncommon. Treatment modalities can include intravenous immunoglobulin, plasma exchange, and corticosteroids. Corticosteroids should be used with caution since they may exacerbate myasthenic symptoms. Treatment with a steroid sparing agent is discussed. A table is presented which lists signs and symptoms that can suggest the need for intubation.

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Myasthenia Gravis: A Review

Autoimmune Diseases ◽

10.1155/2012/874680 ◽

2012 ◽

Vol 2012 ◽

pp. 1-10 ◽

Cited By ~ 25

Author(s):

Annapurni Jayam Trouth ◽

Alok Dabi ◽

Noha Solieman ◽

Mohankumar Kurukumbi ◽

Janaki Kalyanam

Keyword(s):

Myasthenia Gravis ◽

Treatment Modalities ◽

Initial Symptom ◽

Significant Progress ◽

The Past ◽

The Us ◽

Circulating Antibodies ◽

New Treatment ◽

Made In

Acquired myasthenia gravis is a relatively uncommon disorder, with prevalence rates that have increased to about 20 per 100,000 in the US population. This autoimmune disease is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. In about two-thirds of the patients, the involvement of extrinsic ocular muscle presents as the initial symptom, usually progressing to involve other bulbar muscles and limb musculature, resulting in generalized myasthenia gravis. Although the cause of the disorder is unknown, the role of circulating antibodies directed against the nicotinic acetylcholine receptor in its pathogenesis is well established. As this disorder is highly treatable, prompt recognition is crucial. During the past decade, significant progress has been made in our understanding of the disease, leading to new treatment modalities and a significant reduction in morbidity and mortality.

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Antibodies to Acetylcholine Receptors (Ab) and Clinical Condition During Various Treatment Modalities in Myasthenia Gravis: A Follow-up Study

Verhandlungen der Deutschen Gesellschaft für Neurologie - Neuroimmunologie Spinale Krankheiten Neuropsychologie Metabolische Enzephalopathien Neurologische Notfälle Interventionelle Neuroradiologie ◽

10.1007/978-3-642-83201-7_99 ◽

1987 ◽

pp. 427-428

Author(s):

H. J. G. H. Oosterhuis ◽

P. C. Limburg ◽

E. Hummel-Tappel ◽

G. Horst ◽

J. Kuks ◽

...

Keyword(s):

Myasthenia Gravis ◽

Clinical Condition ◽

Acetylcholine Receptors ◽

Treatment Modalities ◽

Follow Up Study

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Various treatment modalities required in myasthenia gravis

Drugs & Therapy Perspectives ◽

10.2165/00042310-199506060-00004 ◽

1995 ◽

Vol 6 (6) ◽

pp. 11-14

Author(s):

&NA;

Keyword(s):

Myasthenia Gravis ◽

Treatment Modalities

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Use Of Intravenous Immunoglobulin And Management Of Anesthesia For Preparation To Thymectomy In A Case With Myasthenia Gravis That Was Intractable To Classical Treatment Modalities

The Internet Journal of Anesthesiology ◽

10.5580/1341 ◽

2009 ◽

Vol 20 (2) ◽

Keyword(s):

Myasthenia Gravis ◽

Intravenous Immunoglobulin ◽

Treatment Modalities

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General: Myasthenia Gravis

Anesthesiology Applied Exam Board Review ◽

10.1093/med/9780190852474.003.0002 ◽

2018 ◽

pp. 11-18

Author(s):

Shimon Frankel

Keyword(s):

Myasthenia Gravis ◽

Disease Severity ◽

Muscle Relaxation ◽

Neuromuscular Disorders ◽

Preoperative Evaluation ◽

Main Topic ◽

Treatment Modalities ◽

Myasthenic Crisis ◽

Crisis Conditions

In this chapter the essential aspects of myasthenia gravis and Eaton-Lambert syndrome are reviewed. The chapter is divided into preoperative, intraoperative, and postoperative sections with important subtopics related to the main topic in each section. Issues addressed with regard to preoperative evaluation include assessment of disease severity, medication for myasthenia gravis, and presence of Lambert-Eaton syndrome. Intraoperative issues discussed are the type of anesthesia used for such patients and dealing with varying degrees of muscle relaxation common to patients with these neuromuscular disorders. Postoperative concerns addressed include postoperative weakness and myasthenic crisis. Conditions that worsen these syndromes are also discussed and treatment modalities are reviewed.

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